Inpatient pain management in sickle cell disease

2019 ◽  
Vol 76 (23) ◽  
pp. 1965-1971 ◽  
Author(s):  
Stefanie M Zassman ◽  
Francis J Zamora ◽  
John D Roberts
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Acute Pain ◽  
Pain Control ◽  
Hospital Admissions ◽  
Healthcare Providers ◽  
Emergency Department Visits ◽  
Cell Disease ◽  
Usual Practice ◽  
Blood Disorder

Abstract Purpose A novel strategy for management of acute pain associated with sickle cell disease (SCD), referred to as the oral tier approach, is described. Summary SCD is an inherited blood disorder characterized by episodic acute pain known as vaso-occlusive crisis (VOC), which is the most common reason for emergency department visits and hospital admissions in patients with SCD; these patients are often treated with parenteral opioids on admission and then transitioned to oral opioids prior to discharge. In this report, experience with use of the oral tier approach in 3 patients with SCD hospitalized for management of VOC is reported. As per usual practice, acute pain was initially managed with parenteral opioids via patient-controlled analgesia (PCA). Once pain control was established, an oral tier was added. The oral tier consisted of 3 orders. The first order was for an oral opioid, to be administered every 3 hours on a scheduled basis; however, the patient could refuse 1 or more of these scheduled doses. Two additional orders specified that the patients could receive additional oral opioids in incremental doses for moderate (grade 4–7) or severe (grade 8–10) pain if appropriate. To facilitate transition to an oral regimen with which the patients might be discharged, they were encouraged to use oral opioids in preference to parenteral opioids. Opioid usage and average daily pain scores for the 3 patients are reported. Conclusion Healthcare providers can use the oral tier approach to facilitate rapid inpatient conversion from i.v. PCA to oral opioids while providing adequate pain control in patients with SCD who develop VOC.

Healthcare injustice in patients with sickle cell disease

2016 ◽  
Vol 9 (2) ◽  
pp. 109-119 ◽  
Author(s):  
Miriam O Ezenwa ◽  
Crystal Patil ◽  
Kevin Shi ◽  
Robert E Molokie
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Pain Control ◽  
Qualitative Interviews ◽  
Healthcare Providers ◽  
Cell Disease ◽  
Content Type ◽  
Patients Experience ◽  
Educational Modules ◽  
Future Work

Purpose – The purpose of this paper is to detail experiences that sickle cell disease (SCD) patients associate with healthcare justice and injustice in pain control. Design/methodology/approach – A content analysis study of open-ended comments written by 31 participants who completed a 20-item healthcare injustice questionnaire-revised twice: once in reference to experiences with doctors and once in reference to experiences with nurses. Findings – Participants’ mean age was 33±10 years; most were African-Americans and women. Themes showed: the four domains of healthcare justice were represented in patients’ comments; examples of justice and injustice were provided; specific incidents and interactions with healthcare providers were memorable to patients; and setting was a factor important to healthcare experiences because expectations about services vary by setting. Research limitations/implications – Patients were self-selected. Future work will include qualitative interviews and focus groups to uncover more details about how patients experience healthcare injustice. Practical implications – Additional training is needed for SCD providers and about proper management of sickle cell pain; educational modules are also needed that address areas of healthcare injustice by patients. Originality/value – The authors are the first to report how patients define healthcare justice and injustice. Specific details about memorable SCD patient-provider interactions and pain control are described.

The Impact of Local Air Quality on the Number of Hospital Admissions with Acute Pain in Sickle Cell Disease within an Urban Environment.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3790-3790
Author(s):  
Deborah Yallop ◽  
Edward R. Duncan ◽  
Ellie Norris ◽  
Gary Fuller ◽  
Nikki Thomas ◽  
...  
Keyword(s):  
Air Quality ◽  
Sickle Cell Disease ◽  
Environmental Factors ◽  
Sickle Cell ◽  
Acute Pain ◽  
Hospital Admissions ◽  
Clinical Severity ◽  
Cell Disease ◽  
Low Levels ◽  
The Impact

Abstract The clinical severity of sickle cell disease (SCD) is dependent on genetic and environmental variables. The impact of environmental factors on disease is a major public health issue and air pollution has been consistently correlated with poor health outcomes. Environmental factors in SCD have been poorly studied. We have retrospectively studied the numbers of daily admissions with vaso-occlusive sickle cell pain to King’s College Hospital, London, in relation to local daily air quality measurements. We analysed 1047 patient episodes over 1400 days (1st January 1998 to 31st October 2001). Statistical time series analysis was performed using cross-correlation function (CCF), where the observations of one series are correlated with the observations of another series at various lags and leads, values >0.05 being significant. This showed a significant association between increased numbers of admissions and low levels of nitric oxide (NO) (CCF=0.063), low levels of carbon monoxide (CO) (CCF=0.064) and high levels of ozone (O3) (CCF=0.067). There was no association with sulphur dioxide (SO2), nitric dioxide (NO2) or dust. The significant results were further examined using quartile analysis. This confirmed that increased numbers of hospital admissions were associated with high levels of O3 (oneway ANOVA p=0.039) and low levels of CO (oneway ANOVA p=0.042). Low NO levels were also associated with increased admissions, not however reaching statistical significance on quartile analysis (oneway ANOVA p=0.158). O3 levels show marked seasonal variation, with high levels occurring in summer months in the UK. We have previously shown a trend towards increased admissions in the summer months with acute sickle related pain, whereas other groups, which are primarily based in rural tropical climates, found increased admissions in cold, rainy months. The adverse effect of high O3 levels may explain this difference. There is no direct evidence to explain the mechanism by which high O3 levels leads to vaso-occlusion in SCD but high levels of O3 have been linked to reduced respiratory function, which may in turn precipitate vaso-occlusion. Independent studies have shown high CO levels are linked to increased respiratory and cardiovascular admissions. Paradoxically we found that high CO levels were linked to decreased admission numbers and may be protective against acute pain in SCD. CO may confer benefit by forming carboxyhaemoglobin that cannot polymerise resulting in decreased sickling. Previous studies have shown prolonged red cell survival in vivo following administration of CO to patients with SCD. Our study also suggests higher levels of atmospheric NO are linked to fewer admissions. NO is known to be central in the pathophysiology of vaso-occlusion and sickle cell patients are thought to have functional deficiency of NO. Many groups have reported inhaled NO as beneficial in the treatment of sickle pain. Our study suggests air quality has a significant effect on acute pain in SCD and that patients should be counselled accordingly. Based on these findings it would be appropriate to warn patients that high O3 levels might precipitate complications of SCD. The potential beneficial effect of CO and NO is intriguing and requires further investigation.

scholarly journals The associations between air quality and the number of hospital admissions for acute pain and sickle-cell disease in an urban environment

2007 ◽  
Vol 136 (6) ◽  
pp. 844-848 ◽  
Author(s):  
Deborah Yallop ◽  
Edward R. Duncan ◽  
Elizabeth Norris ◽  
Gary W. Fuller ◽  
Nikki Thomas ◽  
...  
Keyword(s):  
Air Quality ◽  
Sickle Cell Disease ◽  
Urban Environment ◽  
Sickle Cell ◽  
Acute Pain ◽  
Hospital Admissions ◽  
Cell Disease

Temperature and Humidity Effects on Acute Vaso-Occlusive Pain Episodes in Sickle Cell Disease

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 992-992
Author(s):  
Fuad El Rassi ◽  
Eldrida Randall ◽  
Sidney F. Stein ◽  
Hanna Jean Khoury ◽  
James R. Eckman ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Hospital Admission ◽  
Acute Care ◽  
Sickle Cell ◽  
Hospital Admissions ◽  
Admission Rate ◽  
Emergency Department Visits ◽  
Cell Disease ◽  
Temperature And Humidity ◽  
The City

Abstract Background: Sickle cell patients suffer from painful vaso-occlusive episodes (VOE) that interrupt patients' lives and productivity and lead to emergency department visits and hospitalizations and, on occasion, death. The VOE in sickle cell disease (SCD) accounts for 90% of hospital admissions for patients with SCD and constitute a financial burden on both patients and hospitals. Efforts to prevent pain crises have failed to establish a causal relationship in about 40% of reported cases. The remaining 60% of known causes of VOE are related to dehydration, febrile illness, and infections. The Emory University Sickle Cell Center at Grady Memorial hospital has been providing specialized services for SCD patients for 30 years. The center includes a 24/7 acute care unit (ACU) that is staffed by SCD providers who specialize in the management of VOE. The patients are started on intravenous narcotics and fluids within 30 minutes of presenting to the ACC. After eight hours of management, the patient is then either discharged home if the VOE is controlled or admitted to the hospital for continued management. Annually, around 3000 ACU visits are recorded with a 17% hospital admission rate. When patients are questioned on the reason for their presentation, “weather change” is frequently reported as the trigger of the VOE. A review of the literature reveals scant data to support the hypothesis that weather-related changes trigger sickle cell pain crises. Methods: In a retrospective evaluation of patient visits over the last 4 years, we identified temperature and humidity measurements for that period of time and attempted to correlate them with the frequency of ACU visits. We used the “weatherspark” website that records weather changes for the city of Atlanta because > 90% of our patients reside in the city. Results: The four-year review of weather data and ACU visits did not show a direct correlation between graphs of temperature and humidity, and the number of ACU visits. The annual number of visits to the ACU was 2930, 2467, 3195 and 3370 for the years 2011, 2012, 2013 and 2014 respectively. The average admission rate was 16.6% overall, and the admission rate in the years 2011, 2012, 2013 and 2014 was 12.4%, 14.9%, 19.6% and 19.6% respectively. The attached figure has an example of the year 2013 charts for temperature, humidity, sickle cell acute care visits and hospital admission graphs. Conclusion: The retrospective analysis does not reflect a direct correlation between the temperature and humidity graphs and the number of ACU visits and hospital admissions. Given the volume of data analyzed, it is unlikely that there is any correlation between temperature and humidity variations and either acute care visits or admissions. Atlanta, GA- ACU visits, Hospital admissions, Temperature and Humidity Charts for 2013: DATA from weatherspark.com Figure 1. Figure 1. Figure 2. Figure 2. Figure 3. Figure 3. Disclosures No relevant conflicts of interest to declare.

Procedures, Pain, and Parents

PEDIATRICS ◽  
1991 ◽  
Vol 87 (4) ◽  
pp. 563-565
Author(s):  
HOWARD BAUCHNER
Keyword(s):  
Sickle Cell Disease ◽  
Lumbar Puncture ◽  
Sickle Cell ◽  
Acute Pain ◽  
Cell Disease ◽  
Emergency Rooms ◽  
Pharmacological Agents ◽  
The Past ◽  
The Subject

During the past decade certain types of pain in children have been the subject of much research and discussion. The pain associated with cancer, sickle cell disease, and the preoperative and post-operative periods have all been extensively studied and reviewed.1-4 Less information is available about acute pain inflicted in emergency rooms. Children commonly undergo procedures such as venipuncture, intravenous cannulation, lumbar puncture, and manipulation of fractures in emergency rooms without the benefit of any analgesia. What techniques are available to reduce the pain and anxiety that children feel when they undergo procedures? Traditionally, physicians have tried to reduce pain by using pharmacological agents.

scholarly journals Microfluidics in Sickle Cell Disease Research: State of the Art and a Perspective Beyond the Flow Problem

2021 ◽  
Vol 7 ◽  
Author(s):  
Anupam Aich ◽  
Yann Lamarre ◽  
Daniel Pereira Sacomani ◽  
Simone Kashima ◽  
Dimas Tadeu Covas ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Flow Problem ◽  
Red Cells ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Intercellular Interaction ◽  
Blood Disorder ◽  
Resource Setting ◽  
Wide Range

Sickle cell disease (SCD) is the monogenic hemoglobinopathy where mutated sickle hemoglobin molecules polymerize to form long fibers under deoxygenated state and deform red blood cells (RBCs) into predominantly sickle form. Sickled RBCs stick to the vascular bed and obstruct blood flow in extreme conditions, leading to acute painful vaso-occlusion crises (VOCs) – the leading cause of mortality in SCD. Being a blood disorder of deformed RBCs, SCD manifests a wide-range of organ-specific clinical complications of life (in addition to chronic pain) such as stroke, acute chest syndrome (ACS) and pulmonary hypertension in the lung, nephropathy, auto-splenectomy, and splenomegaly, hand-foot syndrome, leg ulcer, stress erythropoiesis, osteonecrosis and osteoporosis. The physiological inception for VOC was initially thought to be only a fluid flow problem in microvascular space originated from increased viscosity due to aggregates of sickled RBCs; however, over the last three decades, multiple molecular and cellular mechanisms have been identified that aid the VOC in vivo. Activation of adhesion molecules in vascular endothelium and on RBC membranes, activated neutrophils and platelets, increased viscosity of the blood, and fluid physics driving sickled and deformed RBCs to the vascular wall (known as margination of flow) – all of these come together to orchestrate VOC. Microfluidic technology in sickle research was primarily adopted to benefit from mimicking the microvascular network to observe RBC flow under low oxygen conditions as models of VOC. However, over the last decade, microfluidics has evolved as a valuable tool to extract biophysical characteristics of sickle red cells, measure deformability of sickle red cells under simulated oxygen gradient and shear, drug testing, in vitro models of intercellular interaction on endothelialized or adhesion molecule-functionalized channels to understand adhesion in sickle microenvironment, characterizing biomechanics and microrheology, biomarker identification, and last but not least, for developing point-of-care diagnostic technologies for low resource setting. Several of these platforms have already demonstrated true potential to be translated from bench to bedside. Emerging microfluidics-based technologies for studying heterotypic cell–cell interactions, organ-on-chip application and drug dosage screening can be employed to sickle research field due to their wide-ranging advantages.

scholarly journals The gut microbiome in sickle cell disease: Characterization and potential implications

PLoS ONE ◽  
2021 ◽  
Vol 16 (8) ◽  
pp. e0255956
Author(s):  
Hassan Brim ◽  
James Taylor ◽  
Muneer Abbas ◽  
Kimberly Vilmenay ◽  
Mohammad Daremipouran ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Gut Microbiome ◽  
Tissue Injury ◽  
Organ Damage ◽  
Composition Analysis ◽  
Cell Disease ◽  
Blood Disorder ◽  
Major Player ◽  
Next Generation Sequencing Ngs

Background Sickle Cell Disease (SCD) is an inherited blood disorder that leads to hemolytic anemia, pain, organ damage and early mortality. It is characterized by polymerized deoxygenated hemoglobin, rigid sickle red blood cells and vaso-occlusive crises (VOC). Recurrent hypoxia-reperfusion injury in the gut of SCD patients could increase tissue injury, permeability, and bacterial translocation. In this context, the gut microbiome, a major player in health and disease, might have significant impact. This study sought to characterize the gut microbiome in SCD. Methods Stool and saliva samples were collected from healthy controls (n = 14) and SCD subjects (n = 14). Stool samples were also collected from humanized SCD murine models including Berk, Townes and corresponding control mice. Amplified 16S rDNA was used for bacterial composition analysis using Next Generation Sequencing (NGS). Pairwise group analyses established differential bacterial groups at many taxonomy levels. Bacterial group abundance and differentials were established using DeSeq software. Results A major dysbiosis was observed in SCD patients. The Firmicutes/Bacteroidetes ratio was lower in these patients. The following bacterial families were more abundant in SCD patients: Acetobacteraceae, Acidaminococcaceae, Candidatus Saccharibacteria, Peptostreptococcaceae, Bifidobacteriaceae, Veillonellaceae, Actinomycetaceae, Clostridiales, Bacteroidacbactereae and Fusobacteriaceae. This dysbiosis translated into 420 different operational taxonomic units (OTUs). Townes SCD mice also displayed gut microbiome dysbiosis as seen in human SCD. Conclusion A major dysbiosis was observed in SCD patients for bacteria that are known strong pro-inflammatory triggers. The Townes mouse showed dysbiosis as well and might serve as a good model to study gut microbiome modulation and its impact on SCD pathophysiology.

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